Acquired Hemophilia: A Life Threatening Bleeding Disorder

Highlights

  • What is Acquired Hemophilia Disorder
  • Symptoms of Acquired Hemophilia Disorder
  • Causes of Acquired Hemophilia Disorder
  • Diagnosis of Acquired Hemophilia Disorder
  • Various therapies for treating Acquired Hemophilia Disorder
  • Antibodies, Factor VIII, hemostasis, inhibitors, etc.

Summary

Acquired hemophilia is rare and life threatening disease which is caused due to excessive bleeding in the human body. Acquired hemophilia or AH is a rare autoimmune disorder which occurs due to excessive bleeding in the patients or persons who have personal and history of negative hemorrhages in the family. Auto immune disorders like Acquired Hemophilia or AH generally occurs in those human beings whose body’s immune system attacks the healthy tissues or cells of the human body by mistake. In the Acquired hemophilia disorder or AH, the human body produces inhibitors also known as antibodies that mostly attacks or affects the clotting factors of the human body. The body needs the clotting factors which are specialized proteins to clot the blood normally. The person or the individuals those who are suffering from this disease or disorder called as Acquired Hemophilia mainly develops complications like uncontrolled bleeding or abnormal bleeding in the soft tissues, muscles and skin.

If the individual of the person who is suffering from the Acquired Hemophilia or AH is undergoing any surgery, blood clotting will be a major issue and can lead to a following trauma. The symptoms that can specify the Acquired Hemophilia or AH includes epistaxis or normally called as nosebleeds, solid swellings, bruising on the body of the affected individual and also swellings of hematomas also known as congealed blood, one of the most common symptom is hematuria also known as blood in the urine and gastro-intestinal bleeding or urogenital bleeding. Acquired Hemophilia or AH can cause severe and potentially life threatening bleeding complications in some severe cases. The Acquired Hemophilia disorder or AH can be identified in only 50 percent of the cases and the cause for the Acquired Hemophilia is also identifiable and in the remaining 50 percent cases, the cause for the Acquired Hemophilia disorder or AH is unknown.

Introduction

Acquired Hemophilia Disorder or AH is not as same as then congenital hemophilia, these two hemophilia’s are very different from each other. Congenital Hemophilia is caused due to the rare genetic disorders which has occurred because of the congenital deficiency of some of the blood clotting factors. Acquired Hemophilia A disorder or AHA is the main form of hemophilia also known as classic hemophilia. The Acquired Hemophilia A or AHA also called as classic hemophilia is an X-linked disorder that mainly affects or attacks the males but can also affect the females. The Acquired Hemophilia A or AHA is caused because of the deficiency or inactivation of the blood clotting factor VIII. The blood clotting factor VIII is the same blood clotting factor that is mainly affected in the individuals who are suffering from the Acquired hemophilia. Both the disorders mainly involve the deficiency of the same blood clotting factor but the pattern of the bleeding is very much different in both the cases. The reason why the pattern between these two disorders in not understood fully till now.

Acquired hemophilia A is a rare autoimmune disorder caused by an autoantibody (inhibitor) to factor VIII (FVIII) that interferes with its coagulant function and predisposes to severe, potentially life-threatening hemorrhage. Disease management focuses on controlling bleeding, primarily with the use of bypassing therapy and recombinant porcine FVIII, and permanently eradicating the autoantibody using various immunosuppressant.

Symptoms of Acquired Hemophilia (AH)

The major symptoms of the Acquired Hemophilia or AH mostly develops due to the blood that cannot be clotted properly in the human body. Blood clotting is the process in which the blood clumps together to plug at the site of the wound. The most common blood clotting factors which are commonly attacked by the Acquired hemophilia or AH is factor VIII and these factors are specialized proteins that are very important for the blood to clot properly.

Although there are about one of the three patients do not require therapy to control the bleeds and the bleeding severity also varies and this leads to one of the three patients that have multiple bleeding episodes. Ecchymosis also known as subcutaneous bleeding is one of the most common symptom or manifestation of the Acquired Hemophilia or AH which is then followed the muscle bleeding also known as hematoma, melena also known as gastrointestinal, and hematuria also known as genitourinary and retroperitoneal.  The intracranial hemorrhage is very rare but can be fatal in some of the Acquired Hemophilia Disorder or AH. In the case of the congenital Hemophilia A, the joint bleeding is very infrequent.

In the Acquired Hemophilia disorder or AH, bleeding occurs spontaneously or often occurs without cause. In the Acquired Hemophilia disorder or AH, the episodes of bleeding can be more severe and if not treated properly it can become more life-threatening. In some of the patients who are suffering from the Acquired Hemophilia disorder or AH, the bleeding becomes more severe if there is a delay in the diagnosis and in addition some medical issues are present then there are often contributing factors to Acquired Hemophilia disorder or AH.

If the bleeding in the body of the person or the affected patient is more severe than the severity of bleeding leads into the soft tissues and can also progress rapidly and can potentially cause compartmental syndrome. The severity in the bleeding can also cause serious and potentially painful condition by increasing the pressure on the nerves, blood vessels and also on the muscles and more importantly within the legs and arms with severe damage by compression of these structures.

The individuals or the patients who are affected or suffering from the Acquired Hemophilia disorder or AH can be at the risk losing their lives because of the excessive bleeding at the time of the surgery or also at the time of following trauma and sometimes even trivial. The disorder or disease called as the Genital Heavy Bleeding may occur most frequently in the pregnant women at the time of child birth and this genital heavy bleeding is also known as “Postpartum Period”.

 

Causes of Acquired Hemophilia (AH) Disorder

The Acquired Hemophilia is an autoimmune disorder which generally occurs when the inhibitors or the antibodies which are produced by the immune system of the human body that mistakenly attack healthy cells or tissues of the human body and specifically specialized proteins (also known as clotting factors) and the most often clotting factor VIII.

The immune system of the human body normally or generally responds to an outside or a foreign substance by creating or by producing the specifically specialized proteins known as antibodies or inhibitors.  These specifically specialized proteins called as antibodies or inhibitors work by destroying or kills the outside substance or the foreign substances directly or by covering these outside or foreign substances that marks the foreign substances for their destruction by the White Blood Cells (WBS’s).

When the antibodies or inhibitors produced by the immune system of the human body attacks the healthy cells or tissues of the body then those antibodies or inhibitors may be referred as the “autoantibodies”.

Many of the researchers in the world believe that a triggering event (like an underlying disorder or an infection) might lead the immune system to produce autoantibodies. The autoantibodies are also termed as inhibitors in the Acquired Hemophilia disorder or AH because the autoantibodies inhibit the function of the affected clotting factor.

The Acquired hemophilia disorder (AH) is a disease which mainly attacks the weak immune system like elderly individuals or older people. According to the sources, Acquired Hemophilia disorder (AH) mainly attacks old people. Approximately 50 percent of the patients suffering from acquired hemophilia or AH, the disorder or the triggering events of the disease can be identified (also known as idiopathic form) and the remaining 50 percent of the Acquired Hemophilia Patients have conditions or coexisting disorders which may include autoimmune disorders like lupus, multiple sclerosis, sjogren syndrome, infections, diabetes, hepatitis, dermatological or respiratory diseases, Blood cancer (hematological cancer) and some solid tumors or drugs such as penicillin.

Diagnosis

The Acquired Hemophilia disorder (AH) can be suspected or identified only by the clinical picture and the Acquired Hemophilia is confirmed by a test called as Abnormal Coagulation Test. The diagnosis of the patients suffering from Acquired Hemophilia (AH) shall be considered with an onset of abnormal bleeding and a prolongation of the activated partial thromboplastin time (aPTT) and is especially carried for the elderly and post-partum women.

Patients or the individuals suffering from the acquired hemophilia disease (AH) has an isolated prolonged aPTT along with normal PT (Prothrombin Time). Some other tests are also performed to know the other causes of the isolated prolonged aPTT like non-specific inhibitors or therapy like heparin are also performed in some cases.

Some mixing tests are also performed to identify and confirm the Acquired Hemophilia (AH) disease known as aPTT mixing tests. In the aPTT mixing test, the plasma of the affected patient is mixed with normal plasma to further confirm the diagnosis for the Acquired Hemophilia (AH) disease or disorder.

The mixing study mainly differentiates between the factor inhibitors and genetic factor deficiencies. In the mixing studies a sample of blood from the affected patient is taken and mixed with the blood from a controlled subject to identify and confirm the presence of the Acquired Hemophilia disorder or AH.

Standard Therapies and Treatment

As the Acquired Hemophilia (AH) is one of the rarest disorder and hence most of the treatments and therapies that are used to treat the individuals affected by the Acquired Hemophilia (AH) are based upon anecdotal reports or also based on some small cases series. There are some other studies also used to compare the effectiveness of some specific treatments against one another and the treatment is highly individualized.

The procedures for the therapy and interventions to treat the Acquired Hemophilia (AH) disorder will differ from each other depending on the various factors including present specific treatment, overall health and age, the natural course of the disorder, personal preference and other factors. The decisions that has to be taken for the therapy of the Acquired Hemophilia (AH) needs to be taken by the doctor only and also by the health care team under serious consultation by the patients and their families.

Bleeding may be very severe and might have a sudden onset and hence a prompt hemostatic control is mandatory to reduce the mortality of the disease. The International Recommendation states that the anti-hemorrhagic treatment should only be given to those patients who are suffering from severe bleeding and also had been confirmed positive in the diagnosis of Acquired Hemophilia (AH) Disorder. There are two ways available in the treatment of Acquired Hemophilia (AH) disorder:

  • Use of bypassing agents (This concentrates on the factors that bypass the acquired deficiency).
  • Strategies to increase FVIII levels.

The bypassing agent therapy is the recommended first to the patients who are suffering from Acquired Hemophilia (AH) disorder because of their high level of effectiveness and rapid auctioning. The dosage of this treatment or therapy is based on large experience with the management of patients with FVIII inhibitors and is normally based on the clinical assessment.

According to the Global Acquired Hemophilia Treatment Market 2019-2024, the prominent key players or the leading competitors are Baxalta Inc., Genentech Inc., Baxter Healthcare Corporation, Sanofi SA, Bristol-Myers Squibb, Ferring B.V., Novo Nordisk A/S.

, , , ,